A subset of t(11;14) lymphoma with mantle cell features displays mutated IgV(H) genes and includes patients with good prognosis, nonnodal disease
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We analyzed lymphocyte morphology, histology, immunophenotype, immunoglobulin heavy chain (IgV(H)) gene mutations, and clinical course in 80 unselected patients presenting with circulating t(11;14) lymphocytes. Of the 80 patients, 43 had peripheral lymphadenopathy (nodal group), and histology confirmed mantle cell lymphoma (MCL) in all. There were 37 patients with no lymphadenopathy (nonnodal group); 13 of 37 had histology, all showing MCL. IgV(H) genes were unmutated in 28 (90%) of 31 nodal and 15 (44%) of 34 nonnodal cases (P = .0001); CD38 was positive in 32 (94%) of 34 nodal and 16 (48%) of 33 nonmodal cases (P < .001); 41 (95%) of 43 nodal patients required immediate treatment compared with 18 (49%) of 37 nonnodal patients who had indolent disease (P < .0001). Median survival (95% confidence interval) was 30 months (10-50) in the nodal group and 79 months (22-136) in the nonnodal group (P = .005). Mutation status did not statistically affect survival, but of 6 long-term survivors (> 90 months) all were nonnodal and 5 of 5 had mutated IgV(H) genes. Lymphocyte morphology was heterogeneous in both groups: typical MCL in 56 cases (34 nodal, 22 nonmodal), blastoid MCL in 8 cases (3 nodal, 5 nonmodal), and small-cell MCL in 16 cases (6 nodal, 10 nonnodal, P = .12). Matutes immunophenotyping score was 1 in 65 cases and 2 in 15 (8 nodal, 7 nonnodal). We find no evidence against a diagnosis of MCL in the nonnodal group and suggest that mutated IgV(H) genes may help identify patients with indolent disease. (C) 2003 by The American Society of Hematology.
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Molecular Haematology (including Cytogenetics Group and Cell Markers)
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BLOOD, 2003, 101 pp. 4975 - 4981
AMER SOC HEMATOLOGY